RESUMEN
PURPOSE: To identify systemic and/or ophthalmologic predictors of proliferative sickle retinopathy. METHODS: Cross-sectional study comparing clinical, laboratory, and structural choriorretinal aspects between sickle cell disease patients with and without proliferative retinopathy. Patients underwent complete systemic and ophthalmologic evaluation. Enhanced depth spectral domain optical coherence tomography with choroidal binarization and optic coherence tomography angiography were performed and choriorretinal vascular components were compared. RESULTS: Forty-five eyes from 45 sickle cell patients were included. Ninety-one percent of patients were diagnosed with sickle cell retinopathy, 29% with proliferative retinopathy. Mean corpuscular volume, lactate dehydrogenase, and percentage of fetal hemoglobin were reduced in the subgroup of patients with proliferative retinopathy when compared with patients without proliferative retinopathy (p ≤ 0.001; p = 0.04; p ≤ 0.001, respectively). The best predictor of proliferative retinopathy was mean corpuscular volume (AUC = 0.842; p = 0.001), followed by the percentage of fetal hemoglobin (AUC = 0.763, p = 0.009) and lactate dehydrogenase (AUC curve = 0.706; p = 0.039). No differences were found between groups in the quantitative analysis of retinal vascularization using OCTA and choroidal vascularization using OCT (p ≥ 0.05). CONCLUSION: Fetal hemoglobin and mean corpuscular volume may be good predictors of proliferative sickle retinopathy. The association between proliferative retinopathy and reduced levels of lactate dehydrogenase and mean corpuscular volume points to hypoxia and not hemolysis as a possible driving force in its pathophysiology.
RESUMEN
Over the last decade, we have witnessed a massive increase in available clinical agents, both in the clinical trial setting and following commercial use approval, directed to reduced life expectancy as well as the considerable symptom, splenic and anaemia burden associated with myelofibrosis. Given the median age of onset of the disease, coupled with an ageing population globally, we will be caring for an increasingly aged myelofibrosis cohort in future years. We will need to adapt our approach, emphasizing the holistic management of the older individual with myelofibrosis accordingly. Out with the pharmacological management of the disease, consideration needs to be given to interventions based on concurrent illness, comprehensive geriatric assessments, frailty, polypharmacy and drug-drug interactions, nutritional issues, psychological concerns (depression, anxiety or distress), cognitive decline and social/economic aspects. Within this review, we summarise available data addressing these issues, outline knowledge gaps and suggest a summative and holistic approach to the older individual with myelofibrosis.